Surgical Management of Overgrowth Syndrome With Bilateral Vision-Threatening Ptosis.

We describe the first case reported in ophthalmological literature of the surgical management of a 17-month-old boy with bilateral vision-threatening ptosis, tarsomegaly, ectropion, and euryblepharon secondary to suspected overgrowth syndrome. We elaborate on the major challenges associated with surgical management including the natural and asymmetric growth of oversized tissue, the high likelihood of scarring and formation of disorganized tissue, and risks of frequent intubation in these patients who may have lesions that compromise critical structures such as the airway. Ultimately, surgical intervention is encouraged primarily if vision or ocular health is threatened and secondarily to achieve good cosmesis.

Juvenile ocular myasthenia gravis: a report of two cases.

We report 2 cases of pediatric ocular myasthenia gravis. The first case was a 7-year-old girl who presented with bilateral ophthalmoplegia and ptosis that correlated with the onset of upper respiratory symptoms. Neuroimaging and acetylcholine receptor antibody testing were unremarkable. The ice pack test was positive. Symptoms greatly improved with pyridostigmine, with full resolution of ophthalmoplegia achieved by 8-month follow-up. The second case was a 4-year-old girl who presented emergently with ptosis and bilateral ophthalmoplegia. Acetylcholine receptor antibodies testing was positive. The patient was started on pyridostigmine and intravenous immunoglobulin and is scheduled to follow-up with pediatric ophthalmology in the outpatient setting.

Accuracy of Repetitive Ocular Vestibular-Evoked Myogenic Potentials to Diagnose Myasthenia Gravis in Patients With Ptosis or Diplopia.

BACKGROUND AND OBJECTIVES: We developed repetitive ocular vestibular-evoked myogenic potentials (roVEMP) as an electrophysiologic test that allows us to elicit the characteristic decrement of extraocular muscles in patients with ocular myasthenia gravis (OMG). Case-control studies demonstrated that roVEMP reliably differentiates patients with OMG from healthy controls. We now aimed to evaluate the diagnostic accuracy of roVEMP for OMG diagnosis in patients with ptosis and/or diplopia. METHODS: In this blinded prospective diagnostic accuracy trial, we compared roVEMP in 89 consecutive patients presenting with ptosis and/or diplopia suspicious of OMG with a multimodal diagnostic approach, including clinical examination, antibodies, edrophonium testing, repetitive nerve stimulation of accessory and facial nerves, and single-fiber EMG (SFEMG). We calculated the roVEMP decrement as the ratio between the mean of the first 2 responses compared with the mean of the sixth-ninth responses in the train and used cutoff of >9% (unilateral decrement) in a 30 Hz stimulation paradigm. RESULTS: Following a complete diagnostic work-up, 39 patients (44%) were diagnosed with ocular MG, while 50 patients (56%) had various other neuro-ophthalmologic conditions, but not MG (non-MG). roVEMP yielded 88.2% sensitivity, 30.2% specificity, 50% positive predictive value (PPV), and 76.5% negative predictive value (NPV). For comparison, SFEMG resulted in 75% sensitivity, 56% specificity, 55.1% PPV, and 75.7% NPV. All other diagnostic tests (except for the ice pack test) also yielded significantly higher positive results in patients with MG compared with non-MG. DISCUSSION: The study revealed a high sensitivity of 88.2% for roVEMP in OMG, but specificity and PPV were too low to allow for the OMG diagnosis as a single test. Thus, differentiating ocular MG from other neuro-ophthalmologic conditions remains challenging, and the highest diagnostic accuracy is still obtained by a multimodal approach. In this study, roVEMP can complement the diagnostic armamentarium for the diagnosis of MG. CLASSIFICATION OF EVIDENCE: This study provides Class I evidence that in patients with diplopia and ptosis, roVEMP alone does not accurately distinguish MG from non-MG disorders. TRIAL REGISTRATION INFORMATION: ClinicalTrials.gov: NCT03049956.

Blepharoptosis As an Early Manifestation of Neuronal Intranuclear Inclusion Disease.

Neuronal intranuclear inclusion disease (NIID) exhibits diverse clinical manifestations. Our patient was a 64-year-old woman with bilateral ptosis as the chief complaint. She had bilateral miosis, and the pupil was only slightly dilated 60 min after 1% phenylephrine administration, suggesting autonomic dysfunction secondary to preganglionic sympathetic impairment. A head-up tilt test revealed asymptomatic orthostatic hypotension. She was diagnosed with NIID based on a skin biopsy and genetic testing. This study suggests that blepharoptosis is an early manifestation of NIID. Furthermore, patients with suspected NIID should be examined carefully for autonomic dysfunction.

A review of nonsurgical facial rejuvenation and the role of oxymetazoline hydrochloride ophthalmic solution, 0.1% in periorbital revitalization.

BACKGROUND: The demand for nonsurgical facial rejuvenation options is growing, yet the periorbital region remains an area of relative unmet need. This review explores nonsurgical options for facial rejuvenation and the role of oxymetazoline hydrochloride ophthalmic solution, 0.1%, in treating age-related blepharoptosis as part of periorbital rejuvenation. METHODS: Advisors experienced in facial rejuvenation met to discuss existing literature on the upper face and periorbital rejuvenation and the role of oxymetazoline hydrochloride ophthalmic solution, 0.1%, in treating facial aging. RESULTS: An array of nonsurgical options exist to address the signs of aging, including minimally invasive treatments, such as botulinum toxin injections and dermal fillers, and noninvasive therapy, such as lasers, chemical peels, and microdermabrasion. However, treating age-related ptosis in periorbital rejuvenation is mainly addressed surgically. The newly approved α-adrenergic receptor agonist oxymetazoline hydrochloride ophthalmic solution, 0.1%, provides a novel non-interventional approach to blepharoptosis. CONCLUSIONS: Facial rejuvenation is highly sought-after in this post-pandemic era. Each nonsurgical treatment option has its advantages and drawbacks. A patient-centered approach is necessary to select the appropriate procedure considering the patient's concerns and aesthetic sensibilities. The eyes are an area of primary concern for patients, yet surgery is the gold standard for treating ptosis. Oxymetazoline hydrochloride ophthalmic solution, 0.1%, is a safe and effective nonsurgical treatment for blepharoptosis.

Pediatric Ocular Myasthenia Gravis: Single-Center Experience.

BACKGROUND: Currently, there is no universally accepted standard treatment for ocular myasthenia gravis (OMG) in children. We aimed to investigate the possible proper regimens and timing of treatment for pediatric OMG cases based on the clinical manifestations: OMG with ptosis only and OMG with other features. METHODS: One hundred and forty two OMG cases attended at the Department of Pediatrics, Xiangya Hospital, Central South University, from 2010 to 2019 were included, and information from medical records was reviewed and recorded. Comparisons of clinical characteristics between patients with OMG with ptosis only and patients with OMG with other features as well as between patients treated with glucocorticoid (GC) within or after six months from disease onset were performed. RESULTS: OMG with other features constituted about 54.9% of the cases, and 66.2% of the patients achieved optimal outcome. Patients with OMG with ptosis only responded to pyridostigmine alone more than patients with OMG with other features who required several therapies (P < 0.001). Patients with OMG with ptosis only had a larger proportion of optimal outcome than the patients with OMG with other features (P = 0.002), and the difference remained significant even when the individual outcome groups were compared (P < 0.001). Patients who received GC within six months had a greater proportion of optimal outcome than those who received it after six months (P < 0.001). CONCLUSIONS: Although OMG with other features is a more common subtype of OMG, it is also more severe than OMG with ptosis only. An earlier addition of GC leads to optimal outcome.

A simplified technique for correction of complete ptosis secondary to palpebral neurofibromatosis.

PURPOSE: To present a simplified technique in management of complete ptosis secondary to neurofibromatosis. METHODS: This prospective, non-comparative, clinical interventional study included 13 patients with complete ptosis secondary to histologically proved plexiform neurofibromas. It was conducted at the Orbital Unit of Assiut University Hospital, the referral center of Upper Egypt in the period between June 2013 and October 2021. In all cases, a simplified technique of 5 surgical steps was applied: (A) Division of the involved eyelid surgically into three parts by drawing 2 curvilinear lines, the superior line 11 mm below and parallel to the lower eyebrow hairline and the inferior one 10 mm above the lid margin, (B) Resection (full-thickness) of the large middle part which involves the main pathology and lies between the 2 lines, (C) Preservation of the upper part with identification, dissection and clamping of the levator muscle, (D) Refinement of the lower part by removal of any tissue between the skin and the debulked tarsus and (E) Re-suturing of the upper and lower parts in layers; conjunctiva to conjunctiva, levator to tarsus (after resection of a part that corrects the ptosis) and skin to skin. RESULTS: Ptosis was completely corrected in 8 cases (61.5%) and residual mild ptosis occurred in 5 patients (38.5%). No exposure keratopathy or tumor growth was reported during the follow-up period of minimum 1 year. CONCLUSIONS: This simplified technique could be considered as a surgical basis for correction of complete ptosis in neurofibromatosis.

Orbital Fibrotic Band as Cause of Monocular Elevation Deficiency.

Monocular elevation deficiency is a congenital, unilateral inability of elevation. It is classified as innervational, restrictive, or combine types. Here, we report a rare case of monocular elevation deficiency who had 60 PD left hypotropia and left ptosis with limited elevation (-5) both on abduction and adduction. Orbital MRI revealed a hypointense fibrotic band between the superior oblique and superior rectus muscles extending obliquely in the superonasal direction between the sclera and orbital roof. She was successfully treated after severing the fibrotic band between the sclera and bony orbit.

Case Report: Unilateral Hemispheric Hemorrhage with Bilateral Ptosis and Upgaze Palsy.

SIGNIFICANCE: Determining the anatomic location of insult in cases of concurrent bilateral upgaze palsy with bilateral ptosis can be challenging because of the various overlapping pathways and shared functions. It is more commonly related to bilateral oculomotor nerve palsies and myasthenia gravis. However, the possibility of unilateral cerebrovascular events may be overlooked because of the lack of laterality of disease manifestations. PURPOSE: This report documents the uncommon presentation of bilateral ptosis and upgaze palsy in unilateral hemispheric hemorrhage with the corresponding clinical and anatomical review. CASE REPORT: A 46-year-old gentleman presented to the emergency department with left-sided hemiplegia, concurrent bilateral ptosis, and upgaze palsy. He was found to have acute hemorrhagic stroke secondary to significantly elevated blood pressure. Computed tomography of the brain revealed acute extensive intraparenchymal hemorrhage involving the right basal ganglia, frontal lobe, and temporal lobe. There was an extension of hemorrhage into the third ventricle and subarachnoid extension to the Sylvian fissure with obstructive hydrocephalus. An emergency right craniotomy was performed to evacuate the blood clot, and the hydrocephalus subsequently resolved. Post-operatively, bilateral ptosis and upgaze palsy improved and then resolved. CONCLUSIONS: Acute bilateral ptosis and upgaze palsy suggest the possibility of unilateral hemispheric hemorrhage, even though there is no direct involvement of the brainstem and its nuclei.

Severe Upper Lid Fornix Prolapse After a Ptosis Repair in Floppy Eyelid Syndrome: A Case Report.

ABSTRACT: Patients with profound floppy eyelid syndrome (FES) may experience challenges achieving eyelid and tarsal stability in the setting of severe ptosis. A 43-year-old woman with sleep apnea presented to clinic with difficulties driving because of droopy eyelids. She also experienced chronic tearing, crusting, and discharge of the eyes. She was diagnosed with severe FES and significant ptosis. She underwent a wedge excision of the upper lid in the medial and lateral aspect of the upper lid. This was followed by a ptosis repair that resulted in complete upper lid fornix obliteration causing the conjunctiva to obstruct the patient's pupils. The patient's symptoms ultimately improved after a definitive fornix reconstruction. Her visual field symptoms were improved with 1-year follow-up. To our knowledge, this is the first report in the literature of a prolapsed fornix resulting in vision obstruction after floppy eyelid repair via a wedge excision followed by a ptosis repair via an external levator advancement. Given these unusual findings for the patient's age, further investigations into treatment management may be necessary to mitigate eyelid reconstruction complications in younger patients with FES.

[A man with hanging eyelids]. / Een man met hangende oogleden.

A 73-year-old man came to the outpatient clinic because of diplopia and hanging eyelids. Neurological examination revealed fatigueable bilateral ptosis. The patient had a strong positive ice pack-test. Myasthenia gravis preached on top of the differential diagnosis. Subsequent blood tests showed antibodies against ACh-receptors and confirmed the diagnosis.

Efficacy of apraclonidine eye drops in treating ptosis secondary to myasthenia gravis: A pilot clinical trial.

INTRODUCTION/AIMS: Most patients with myasthenia gravis (MG) develop ocular manifestations during their illness and up to 22% may have isolated ocular myasthenia gravis (OMG). Apraclonidine elevates the eyelid by activating alpha-2 receptors on Muller's muscle, an accessory eyelid elevator muscle. In this study we evaluate the effect of apraclonidine in alleviating ptosis secondary to MG. METHODS: This clinical trial (NCT05045248) was done at the American University of Beirut Medical Center. Patients with ptosis secondary to MG were administered two drops of apraclonidine 0.5% solution to the most affected eye. We measured palpebral fissure height (PF), marginal reflex distance-1 (MRD1), marginal reflex distance-2 (MRD2), and levator function (LF) before drug administration and at 1, 5, 30, and 60 minutes after administration. RESULTS: Ten participants were enrolled in the study. Improvement in all eyelid measurements was noted in all participants as early as 1 minute after apraclonidine administration. From baseline to 60 minutes after administration, average PF increased from 8.8 ± 1.9 mm to 14.2 ± 2.6 mm, MRD-1 from 1.7 ± 1.4 mm to 5.4 ± 2.9 mm, MRD-2 from 7.1 ± 1.3 mm to 8.8 ± 1.7 mm, and LF from 13.4 ± 2.9 mm to 17.5 ± 2.4 mm. All increases were statistically significant. DISCUSSION: Apraclonidine may alleviate ptosis secondary to MG and may be an effective alternative treatment for this group of patients.

[Research progress of iatrogenic blepharoptosis repair after double eyelid surgery].

Objective: To summarize the etiology mechanism and treatment of iatrogenic blepharoptosis after double eyelid surgery in Asia. Methods: To extensively review the literature related to iatrogenic blepharoptosis after double eyelid surgery, and to summarize and analyze the related anatomical mechanism, existing treatment options, and indications. Results: Iatrogenic blepharoptosis is a relatively common complication after double eyelid surgery, sometimes it is combined with other eyelid deformities such as sunken upper eyelid and wide double eyelid, which makes it difficult to repair. The etiology is mainly caused by improper adhesion of tissues and scars, improper removal of upper eyelid tissue, and injury of a link of levator muscle power system. Whether blepharoptosis occurs after double eyelid surgery by incision or suture, it should be repaired by incision. The principles of repair include surgical loosening of tissue adhesion, anatomical reduction, and repair of damaged tissues. The key is to use surrounding tissues or transplanted fat to prevent adhesion. Conclusion: When repairing iatrogenic blepharoptosis clinically, appropriate surgical methods should be selected based on the causes and severity of the blepharoptosis, combined with treatment principles, in order to achieve better repair results.

Congenital Orbital Fibrosis With Spontaneous Regression of Orbital Tumor.

Congenital orbital fibrosis (COF) is a rare disorder characterized by an infiltrating orbital mass with secondary involvement of the extraocular muscles that may present with extraocular muscle dysfunction, and globe and eyelid abnormalities in infancy. This condition is thought to be a nonprogressive process and literature on longitudinal assessment of COF is limited. The authors describe a case of COF which was followed for 15 years. The patient had stable symptoms of ocular dysmotility and ptosis but was noted to have spontaneous regression of the orbital mass on serial MRI.

Orbital Schwannoma With Frontal Bone Lysis.

Orbital schwannoma is an exceptionally rare cause of ptosis. Diagnosis may be elusive given its slow rate of growth and its various presentations depending on localization. Herein, we report the case of a 50-year-old male who presented to our clinic with a complaint of unilateral, recurrent ptosis of the left eye. He underwent levator palpebrae resection, which was unsuccessful at improving his ptosis. He later represented with acute-onset diplopia for which magnetic resonance imaging was obtained. Magnetic resonance imaging showed a lesion in the superior orbit with secondary bony dehiscence of the orbital roof. Through a vertical lid-split incision, the lesion was removed, and the frontal lobe was observed protruding through the defect in the orbital roof. This case highlights the importance of diagnostic skepticism in the face of recurrent ptosis and emphasizes the utility of the vertical lid-split approach for anterior lesions of the superior orbit.

Incidence of Blepharoptosis After Pars Plana Baerveldt 350 Glaucoma Implant Surgery by a Single Surgeon.

PURPOSE: To investigate the incidence of postoperative blepharoptosis and clinical risk factors for blepharoptosis after pars plana Baerveldt 350 glaucoma implantation (BGI) by a single surgeon for refractory glaucoma. METHODS: Twenty-four patients (30 eyes) who underwent pars plana BGI for refractory glaucoma at Toyama University Hospital between November 2019 and February 2021 were included. Patients with a preoperative margin reflex distance-1 (MRD-1) of ≥2 mm were included in the study, and a decrease in MRD-1 of ≥2 mm at 6 months postoperatively was defined as blepharoptosis. RESULTS: The mean MRD-1 decreased significantly from 3.2 ± 0.6 mm preoperatively to 2.4 ± 1.1 postoperatively ( p < 0.01). Postoperative ptosis developed in 8 eyes (26.7%). A comparison of the ptosis (n = 8) and nonptosis (n = 22) groups showed a significant difference in the history and number of previous filtration surgeries ( p = 0.02 and p = 0.03, respectively). Those with previous filtration surgery had a higher risk of blepharoptosis after pars plana BGI compared with those without previous filtration surgery (OR: 6.43; 95% confidence interval: 1.03-40.26; p = 0.04). CONCLUSION: Pars plana BGI is a risk factor for postoperative blepharoptosis. Particular attention should be paid to eyes that have undergone previous filtration surgery.

Müller Muscle-Conjunctival Resection for Treatment of Contralateral Ptosis following Unilateral External Levator Advancement.

BACKGROUND: One of the main causes of unsatisfactory outcomes after unilateral blepharoptosis surgery is asymmetry of the upper eyelid height, which occurs as a result of a contralateral eyelid droop. Therefore, the authors evaluated the efficacy of Müller muscle-conjunctival resection (MMCR) for the treatment of contralateral ptosis following unilateral external levator advancement (ELA). METHODS: This study analyzed 26 eyelids of 26 patients with upper eyelid height asymmetry following unilateral ELA who underwent contralateral MMCR retrospectively. The phenylephrine test was performed before ELA and before MMCR. The main outcome measures were symmetry outcomes and clinical outcomes. RESULTS: The mean patient age was 55.81 ± 7.98 years (range, 44 to 70 years); 15 were female (57.7%). The Hering dependency was observed in 13 of the patients (50%) before ELA. An adequate response to phenylephrine was observed before MMCR but not before ELA. Symmetry outcomes after MMCR were perfect (<0.5 mm), good (≥0.5 mm and <1 mm), and fair (≥1 mm) in seven, 17, and two patients, respectively. An optimal upper eyelid height was noted in 47 of the 52 eyelids after the MMCR, whereas three of the 52 eyelids had minimal overcorrection, and two eyelids had undercorrection. The mean change in marginal reflex distance 1 of the contralateral eyelid droop was greater for patients with than without the Hering dependency ( P < 0.0001) after ELA but not after MMCR. Two patients (7.6%) underwent revision ELA surgery. CONCLUSION: MMCR and use of the phenylephrine test to predict the eyelid position may represent an alternative approach in patients who require management of contralateral ptosis following unilateral ELA. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Ophthalmologic clinical features of ocular myasthenia gravis.

To investigate the clinical features of ocular myasthenia gravis (OMG) in ophthalmology. A total of 28 patients with ptosis or diplopia who were followed for at least 6 months between March 2016 and February 2022 were included in this study. The clinical symptoms of the patients and test results were analyzed. According to the positivity of serologic or electrophysiologic test, these patients were divided into 2 groups (positive and negative OMG results) and according to the clinical symptoms of diplopia or ptosis for comparison. Ptosis, diplopia, and both ptosis and diplopia were present in 6 (21.43%), 14 (50.0%), and 8 (28.57%) patients, respectively. Acetylcholine receptor auto-antibody (AchR Ab) was positive in 16 (57.14%) of 28 patients and the ice test was positive in 13 (92.86%) of 14 patients with ptosis. Abnormal thymic lesions were presented in 7 (25.0%) patients, and a definite improvement in response to pyridostigmine was observed in 27 (100.0%) patients. Both ptosis and diplopia were significantly higher in the group with positive results than that in the negative results group (P = .025). In addition, both horizontal and vertical diplopia was significantly higher in the group with AchR Ab titer > 5.0 than that in the group with AchR Ab titer < 5.0 (P = .041). After excluding cranial nerve palsy, if there is ptosis and diplopia, especially vertical diplopia, the possibility of OMG should be considered.

Prognostic predictors of remission in ocular myasthenia gravis.

BACKGROUND: Ocular myasthenia gravis (OMG) constitutes 15% of all myasthenia gravis patients. METHODS: One hundred eight patients with OMG followed-up for over 36 months were retrospectively evaluated regarding factors associated with remission. Demographic features, neuro-ophthalmologic findings at onset, acetylcholine receptor (AChR Ab) and muscle-specifc tyrosine kinase antibodies (MuSK Ab), thymic status, single fiber electromyography (SFEMG) results were the variables considered. RESULTS: Median age of disease onset was 57 years (range 18-82 years). Clinical features at onset was isolated ptosis in 55 (50.9%) and isolated diplopia in 33 (30.6%) patients. Combined ptosis and diplopia were present in 20 (18.5%) patients. Among 75 patients with ptosis, it was unilateral in 65 (86.7%) and bilateral in 10 (13.3%). AChR Abs were found in 66 (61.1%) and MuSK Abs in 2 (1.9%) patients. SFEMG abnormality was detected in 74 (68.5%) patients. Thymoma was present in 16 (14.8%) and thymic hyperplasia in 6 (5.6%) patients. Forty-one patients (37.9%) had been treated with pyridostigmine alone. Sixty-seven (62%) patients were given immunosupressive drugs. In 53 (49.1%) prednisone was used and in 14 (12.9%) patients it was combined with azathioprine. Thymectomy was performed in all 16 patients with thymoma. Complete stable remission (CSR) was achieved in 49 (45.4%) patients. Fifty-nine (54.6%) patients had reached minimal manifestation (MM) status; 32 (29.6%) having a status of MM-1 and 27 (25%) a status of MM-3. CONCLUSIONS: The presence of AchR Abs (p = 0.034) and an abnormal SFEMG (p = 0.006) at onset as increased risk factors for the presence of ongoing signs necessitating medical treatment.

Orbital Inflammatory Pseudotumor following mRNA COVID-19 Vaccination.

The authors present a case of orbital pseudotumor after mRNA COVID-19 vaccination. A 40-year-old otherwise healthy woman was referred to our oculoplastics unit because of left blepharoptosis of 2 months duration starting 1 week after she received her first Pfizer-BioNTech mRNA vaccination. On presentation, her best-corrected visual acuity was 20/20 in each eye. The external examination revealed left blepharoptosis and mild upper eyelid swelling. Orbital magnetic resonance imaging revealed left lacrimal gland enlargement with homogeneous contrast enhancement and diffuse mild enlargement of the left lateral and superior rectus muscles. The results of the extended workup for autoimmune and infectious etiologies and the systemic examination findings were normal. Systemic corticosteroids were started for the orbital pseudotumor. The presented case of orbital pseudotumor development after the mRNA vaccine may be considered to be an immunological process targeting the orbital tissue following immunization, although the cause-effect relationship remains uncertain.